{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T09:02:23Z","timestamp":1777366943732,"version":"3.51.4"},"reference-count":12,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2017,3,22]],"date-time":"2017-03-22T00:00:00Z","timestamp":1490140800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J of Rheum Dis"],"published-print":{"date-parts":[[2018,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Macrophage activation syndrome (<jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content>) is one of the serious complications associated with rheumatic diseases, especially systemic juvenile idiopathic arthritis (<jats:styled-content style=\"fixed-case\">sJIA<\/jats:styled-content>). Here we describe a 9\u2010year\u2010old girl with rheumatoid factor (<jats:styled-content style=\"fixed-case\">RF<\/jats:styled-content>)\u2010positive polyarticular <jats:styled-content style=\"fixed-case\">JIA<\/jats:styled-content>, not <jats:styled-content style=\"fixed-case\">sJIA<\/jats:styled-content>, combined with pneumonia who was successfully treated by plasma exchange. She was diagnosed with <jats:styled-content style=\"fixed-case\">RF<\/jats:styled-content>\u2010positive polyarticular <jats:styled-content style=\"fixed-case\">JIA<\/jats:styled-content> based on positive <jats:styled-content style=\"fixed-case\">RF<\/jats:styled-content> and multiple joint swelling and tenderness 3 years ago. She was admitted in our hospital with myalgia for 2 days and a high fever for half a day. Physical examination revealed relapsing joints symptoms and rough breathing sounds of lungs. The laboratory examination showed increased liver enzymes, elevated serum ferritin and procalcitonin (<jats:styled-content style=\"fixed-case\">PCT<\/jats:styled-content>), decreased percentage of nature killer (<jats:styled-content style=\"fixed-case\">NK<\/jats:styled-content>) cells and fibrinogen, and activated macrophage phagocytosing hematopoietic elements in bone marrow. The elevated <jats:styled-content style=\"fixed-case\">PCT<\/jats:styled-content> and chest computed tomography scan confirmed she also had pneumonia. Intravenous methylprednisolone and oral cyclosporine A followed by intravenous immunoglobulin were added on the basis of antibiotics therapy, but clinical symptoms and laboratory findings did not improve. Finally, we changed to plasma exchange once every other day for a total of three times. Within 1 week, the girl recovered from the <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> completely.<\/jats:p>","DOI":"10.1111\/1756-185x.13064","type":"journal-article","created":{"date-parts":[[2017,3,23]],"date-time":"2017-03-23T07:53:01Z","timestamp":1490255581000},"page":"1142-1145","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Plasma exchange successfully treated macrophage activation syndrome in rheumatoid factor\u2010positive polyarticular juvenile idiopathic arthritis with co\u2010existing pneumonia"],"prefix":"10.1111","volume":"21","author":[{"given":"Lianjie","family":"Shi","sequence":"first","affiliation":[{"name":"Department of Pediatrics West China Second University Hospital Sichuan University Chengdu China"},{"name":"Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University) Ministry of Education Chengdu China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fanlei","family":"Hu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology Peking University People's Hospital Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chuanhui","family":"Xu","sequence":"additional","affiliation":[{"name":"Department of General Medicine Tan Tock Seng Hospital Singapore Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Huaqun","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology Peking University People's Hospital Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Di","family":"Qie","sequence":"additional","affiliation":[{"name":"Department of Pediatrics West China Second University Hospital Sichuan University Chengdu China"},{"name":"Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University) Ministry of Education Chengdu China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chuanjie","family":"Yuan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics West China Second University Hospital Sichuan University Chengdu China"},{"name":"Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University) Ministry of Education Chengdu China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuhong","family":"Tao","sequence":"additional","affiliation":[{"name":"Department of Pediatrics West China Second University Hospital Sichuan University Chengdu China"},{"name":"Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University) Ministry of Education Chengdu China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hanmin","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics West China Second University Hospital Sichuan University Chengdu China"},{"name":"Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University) Ministry of Education Chengdu China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,3,22]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(85)80072-X"},{"key":"e_1_2_4_3_1","first-page":"623","article-title":"Macrophage activation syndrome and etanercept in children with systemic juvenile rheumatoid arthritis","volume":"31","author":"Sawar H","year":"2004","journal-title":"J 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